Seventy percent were reported in the past 6 years A statistical analysis of all medical literature on lymphangioleimyomatosis (LAM) in China from 1981.1.1 to 2009.12.31 showed that the cumulative number of reported cases of LAM in China has exceeded 100. The first case of LAM in China was reported in 1981, and the number of reported cases of LAM has gradually increased since then. Of all 111 female patients with more complete data and meeting the European Society of Respiratory Diseases criteria for a confirmed or proposed diagnosis of LAM, 72% were reported in the past 6 years (2004-2009). A total of 18 provinces in the country reported LAM cases. All of these patients were female, and the median age of onset was only 34 years. Common symptoms included dyspnea (93.7%), pneumothorax (48.6%), cough (45.7%), hemoptysis (35.1%) and celiac disease (33.3%). LAM occurred in 11 patients (9.9%) with tuberous sclerosis complex (TSC). High-resolution CT of the chest showed diffuse cystic lesions in both lungs (104/109). 102 cases (91.9%) were diagnosed by pathological examination. The data showed that the diagnosis rate of rare disease LAM has been greatly improved in recent years due to the application of high-resolution chest CT and the increase of diagnostic awareness. Introduction to pulmonary lymphangioleiomyomatosis (LAM): LAM is a rare disease that occurs mainly in women of childbearing age and is poorly understood by many physicians. It can be disseminated or associated with the genetic disease tuberous sclerosis (TSC). The incidence of disseminated LAM in adult women is approximately 1 in 400,000. patients with LAM usually have progressive dyspnea and recurrent episodes of pneumothorax and celiac disease. Extra-pulmonary manifestations such as renal angiomyolipoma are common. LAM is often misdiagnosed or missed clinically because it is poorly understood. The diagnosis should be highly suspected by HRCT of the chest with characteristic diffuse multiple, mostly 2C5 mm diameter, thin-walled cystic changes in both lungs and can be confirmed by biopsy if necessary. There is still a lack of definitive and effective treatment. Severe cases require lung transplantation. Clinical awareness of this rare disease is called for, and collaboration should be strengthened to make full use of the limited number of cases to conduct effective clinical studies and promote the improvement of diagnosis and treatment as soon as possible.